title: Pancreatic mucinous cystic tumor in Turner syndrome: How a tumor bends to a genetic disease.
abstract: BACKGROUND
  Mucinous cystic neoplasms (MCN) are uncommon tumors of the pancreatic corpus/tail occurring mostly in middle-aged women, with a variable clinico-biological behavior. On histology, MCNs concurrently show an epithelial mucosecreting component with ovarian-type stromal cells.
  
  
  METHODS
  This report describes the first case of a pancreatic MCN with no ovarian-type stroma in a patient with Turner syndrome (TS).
  
  
  CONCLUSIONS
  The mesenchymal component of MCN presumably results from the intra-pancreatic entrapment of ovarian stroma during embryogenesis. In our case, the absence of such stromal component may relate to the "dysgenetic" changes in the ovary involved in TS.
  
  
  CONCLUSIONS
  The present case of primary pancreatic MCN arising in a TS-patient triggers some original speculation on the morphogenesis of pancreatic MCN, also expanding the current clinico-pathological knowledge of this extremely rare entity.
authors.affiliation: Department of Medicine DIMED, Surgical Pathology & Cytopathology Unit, University of Padova, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Pizzi
authors.forename: Gianmaria
authors.initials: G
authors.lastname: Pennelli
authors.forename: Isabella
authors.initials: I
authors.lastname: Merante-Boschin
authors.forename: Matteo
authors.initials: M
authors.lastname: Fassan
authors.forename: Maria Rosa
authors.initials: MR
authors.lastname: Pelizzo
authors.forename: Massimo
authors.initials: M
authors.lastname: Rugge
keywords: Cystadenocarcinoma; Cystadenoma; IPMN; MCN; Pancreatic mucinous cystic neoplasm; SPNP; TS; Turner syndrome; US; intraductal papillary mucinous neoplasm; mucinous cystic neoplasm; solid-pseudopapillary neoplasm of the pancreas; ultrasound echography